60:
323:
277:(PCP), which is common in infants with hyper IgM syndrome, is a serious illness. PCP is one of the most frequent and severe opportunistic infections in people with weakened immune systems. Many CD40 Ligand Deficiency are first diagnosed after having PCP in their first year of life. The fungus is common and is present in over 70% of healthy people's lungs, however, Hyper IgM patients are not able to fight it off without the administration of
2582:
2612:
2622:
1358:
2602:
259:
34:
2592:
473:
in males. Limited data is available on the frequency of AICDA deficiency, another subtype of X-HIGM, but it is believed to affect less than 1 in 1,000,000 individuals. Globally, all forms of HIGM make up approximately 0.3% to 2.9% of all patients diagnosed with primary immunodeficiency disorders (PIDs).
349:
efficiently switch to other types of antibodies as needed to attack invading bacteria, viruses, and other pathogens. In people with hyper IgM syndromes, the B cells keep making IgM antibodies because they can't switch to a different antibody. This results in an overproduction of IgM antibodies and an
472:
All forms of hyper-IgM syndrome are rare. According to the US X-HIGM registry, the prevalence of X-linked hyper IgM syndrome (X-HIGM) during the period from 1984 to 1993 was approximately 1 in 1,000,000 live births. The estimated frequency of CD40L deficiency, a subtype of X-HIGM, is 2 in 1,000,000
832:
Leven, Emily A.; Maffucci, Patrick; Ochs, Hans D.; Scholl, Paul R.; Buckley, Rebecca H.; Fuleihan, Ramsay L.; Geha, Raif S.; Cunningham, Coleen K.; Bonilla, Francisco A.; Conley, Mary Ellen; Ferdman, Ronald M.; Hernandez-Trujillo, Vivian; Puck, Jennifer M.; Sullivan, Kathleen; Secord, Elizabeth A.
250:
Heterozygous females in X-linked hyper IgM syndrome (HIGM1) are usually asymptomatic. However, immunologic testing has revealed that they exhibit reduced expression of CD40L when CD4+ T lymphocytes are activated. In some cases, females with significant reduction in circulating lymphocytes carrying
237:
The majority of patients with HIGM syndrome present with a broad spectrum of clinical symptoms even with a same genetic defects. They usually develop symptoms in infancy and second year of life, including increased susceptibility to infections by extracellular bacteria, sinus & ear infections,
1142:
Vavassori, Valentina; Mercuri, Elisabetta; Marcovecchio, Genni E; Castiello, Maria C; Schiroli, Giulia; Albano, Luisa; Margulies, Carrie; Buquicchio, Frank; Fontana, Elena; Beretta, Stefano; Merelli, Ivan; Cappelleri, Andrea; Rancoita, Paola MV; Lougaris, Vassilios; Plebani, Alessandro (5 March
451:
The primary goal is to address the underlying defect in CD40L or other gene mutations causing HIGM. The potential for precise correction of the CD40LG gene in T cells and hematopoietic stem/progenitor cells (HSPC) to treat X-linked hyper-IgM Syndrome (HIGM1) is a promising avenue of research.
452:
However, the actual therapeutic efficacy of this approach is not yet fully understood and requires further investigation to determine its true potential. In addition to HSCT, supportive measures are crucial for managing infections and complications associated with HIGM. This may include
1297:
1282:
410:. In females, the diagnosis of HIGM1 is extremely rare. Heterozygous females are usually asymptomatic unless there is skewed X-chromosome inactivation. The diagnosis of hyper IgM syndrome can be done via the following methods and tests:
1404:
890:
Lanzi, Gaetana; Ferrari, Simona; Vihinen, Mauno; Caraffi, Stefano; Kutukculer, Necil; Schiaffonati, Luisa; Plebani, Alessandro; Notarangelo, Luigi
Daniele; Maria Fra, Anna; Giliani, Silvia (23 December 2010).
405:
The diagnosis of X-linked hyper IgM syndrome (HIGM1) is established in males with typical clinical and laboratory findings by identifying a hemizygous pathogenic variant in the CD40LG gene through molecular
115:
They are resulting from mutations in the pathway from B-cell activation to isotype class switching. Patients with HIGM are usually diagnosed within the first two years of life and experience severe
389:
and antigen presenting cells (APCs) is known as the underlying cause of HIGM syndromes. CD40L-CD40 interaction is the first step in B cell stimulation for class switch recombination (CSR) and
1397:
1390:
206:
caused by the mutation in the NEMO (nuclear factor ÎșB essential modulator) complex, which, when mutated, is unable to phosphorylate IÎșB downstream of CD40 signaling.
2651:
119:. This syndrome is also known as immunoglobulin class switch recombination (Ig-CSR) deficiencies. The most common causes are mutations in the CD40 Ligand (
2111:
1622:
1470:
456:, immunoglobulin replacement therapy, and close monitoring of respiratory and gastrointestinal infections. Additionally, anti-microbial therapy, use of
2452:
1659:
723:
Yazdani, Reza; Fekrvand, Saba; Shahkarami, Sepideh; Azizi, Gholamreza; Moazzami, Bobak; Abolhassani, Hassan; Aghamohammadi, Asghar (1 January 2019).
2051:
1786:
2646:
1488:
457:
2351:
951:
Dunn, Clinton P.; de la Morena, M. Teresa (1993), Adam, Margaret P.; Mirzaa, Ghayda M.; Pagon, Roberta A.; Wallace, Stephanie E. (eds.),
1791:
2487:
1829:
1243:
1215:
1145:"Modeling, optimization, and comparable efficacy of T cell and hematopoietic stem cell gene editing for treating hyperâIgM syndrome"
2605:
1726:
1643:
1554:
487:
252:
397:
is affected. Certain insults, usually from encapsulated bacteria and toxin, then have a greater opportunity to damage the body.
2477:
1911:
1796:
1666:
1538:
187:
151:
278:
1533:
2457:
2101:
1233:
1646:
1498:
1465:
1205:
482:
2081:
169:
1612:
2510:
1632:
1527:
1522:
1517:
1512:
1507:
1442:
224:
210:
202:
176:
158:
136:
2302:
2224:
2219:
2139:
1899:
1681:
1429:
1312:
220:
gene. UNG is responsible for the cleavage of cytosines that have been deaminated by AID in single-stranded DNA.
109:
1382:
2656:
2282:
2256:
1781:
1766:
1762:
1373:
425:
394:
239:
1874:
1822:
775:"Class-Switch Recombination (CSR)/Hyper-IgM (HIGM) Syndromes and Phosphoinositide 3-Kinase (PI3K) Defects"
453:
271:
216:
112:
characterized by low or absent levels of serum IgG, IgA, IgE and normal or increased levels of serum IgM.
251:
the CD40L mutation due to skewed X-chromosome inactivation can present with symptoms resembling HIGM1 or
228:
is the least-characterized of the HIGM types, as the gene is unknown. Resembles HIGM2, but AID is normal.
2525:
2411:
2384:
2357:
2309:
2263:
1958:
1756:
1736:
1457:
725:"The hyper IgM syndromes: Epidemiology, pathogenesis, clinical manifestations, diagnosis and management"
436:
Flow
Cytometry (evaluate the presence and function of certain immune cells, such as T cells and B cells)
390:
191:
892:
724:
2545:
2505:
2372:
2186:
2181:
2161:
2096:
1948:
1776:
1480:
194:
with CD40L deficiency but B cells from CD40 deficient patients are unable to undergo class switching
59:
2625:
2567:
2550:
2209:
1926:
1301:
154:. Therefore, the only antibody secreted by the B cell is IgM, the least specific class of antibody.
2611:
2515:
2472:
2462:
2251:
2204:
2171:
2166:
1916:
1894:
930:
752:
461:
243:
68:
1091:
773:
Jhamnani, Rekha D.; Nunes-Santos, Cristiane J.; Bergerson, Jenna; Rosenzweig, Sergio D. (2018).
2585:
2467:
2437:
2416:
2379:
2362:
2277:
2241:
2231:
2126:
2046:
1990:
1953:
1938:
1879:
1846:
1815:
1597:
1588:
1573:
1417:
1239:
1211:
1182:
1164:
1072:
1064:
1023:
1005:
960:
922:
914:
872:
854:
814:
796:
744:
692:
116:
86:
48:
186:
gene and it is inherited by autosomal recessive manner. It has similar phenotype of impaired
150:
results in defective signaling to B cells, which do not receive the needed signal to undergo
2287:
2272:
2149:
2024:
1980:
1695:
1676:
1607:
1421:
1413:
1172:
1156:
1054:
1013:
997:
904:
862:
846:
804:
786:
736:
355:
322:
41:
518:"X-linked Immunodeficiency With Hyper IgM Clinical Presentation: History, Physical, Causes"
2615:
2560:
2421:
2406:
2389:
2329:
2324:
2268:
2246:
1943:
1889:
1869:
1718:
407:
382:
1363:
517:
2447:
2401:
2214:
2144:
1963:
1771:
1671:
1493:
1306:
1177:
1144:
1059:
1042:
1018:
985:
867:
834:
809:
774:
310:
295:
2640:
2530:
2319:
2314:
2176:
2116:
2019:
1001:
386:
330:
Different genetic defects cause HIgM syndrome, the vast majority are inherited as an
934:
756:
2595:
2535:
2520:
2346:
2071:
1921:
1906:
1852:
1747:
1559:
1341:
1317:
909:
2394:
2339:
2236:
2091:
2066:
1884:
442:
Blood test(Immunoglobulin levels, Antibody response, Complete blood count (CBC))
420:
300:
2482:
740:
255:. Among the presentation consistent with hyper IgM syndrome are the following:
2367:
2334:
2106:
2056:
2041:
2036:
2029:
2014:
2004:
1752:
1713:
1291:
850:
684:
430:
247:
53:
1168:
1068:
1009:
952:
918:
858:
800:
791:
748:
2555:
2297:
2292:
2156:
2134:
2009:
1973:
1336:
1160:
305:
283:
267:
1186:
1076:
1027:
964:
926:
876:
818:
696:
683:
Johnson, Judith; Filipovich, Alexandra H.; Zhang, Kejian (1 January 1993).
198:
upon activation with agonists and cytokines as per their intrinsic defect .
258:
2061:
1838:
1703:
1438:
893:"Different molecular behavior of CD40 mutants causing hyper-IgM syndrome"
342:
338:
331:
290:
195:
168:
gene. In this type, B cells cannot recombine genetic material to change
1274:
627:"OMIM Entry â # 608106 â IMMUNODEFICIENCY WITH HYPER-IgM, TYPE 5; HIGM5"
599:"OMIM Entry â # 606843 â IMMUNODEFICIENCY WITH HYPER-IgM, TYPE 3; HIGM3"
574:"OMIM Entry â # 605258 â IMMUNODEFICIENCY WITH HYPER-IgM, TYPE 2; HIGM2"
546:"OMIM Entry â # 308230 â IMMUNODEFICIENCY WITH HYPER-IgM, TYPE 1; HIGM1"
393:(SHM) resulting in the generation of various Ig isotypes. Consequently,
2540:
2000:
1968:
1861:
346:
123:) gene located at Xq26.3-27 leading to X-linked HIGM (XHIGM) in males.
33:
652:"OMIM Entry â 608184 â IMMUNODEFICIENCY WITH HYPER-IgM, TYPE 4; HIGM4"
1578:
1447:
1286:
374:
147:
142:
1117:
651:
598:
545:
378:
257:
164:
370:
182:
1811:
1386:
626:
573:
414:
359:
351:
334:
recessive genetic trait and most with the condition are male.
1207:
Immunology: Clinical Case
Studies and Disease Pathophysiology
1807:
238:
skin infections. Furthermore, these patients are prone to
131:
Five types of hyper IgM syndrome have been characterized:
984:
Davies, E Graham; Thrasher, Adrian J (27 November 2016).
162:(autosomal recessive), characterized by mutations of the
835:"Hyper IgM Syndrome: a Report from the USIDNET Registry"
341:
that all B cells produce initially before they undergo
246:, hematologic abnormalities, lymphoproloferation and
146:
gene. In this type, lack of CD40L on the surfaces of
1264:
2498:
2436:
2197:
2125:
2080:
1989:
1860:
1845:
1735:
1712:
1694:
1642:
1572:
1547:
1479:
1456:
1437:
1428:
1327:
1268:
959:, Seattle (WA): University of Washington, Seattle,
95:
85:
77:
67:
47:
26:
345:due to exposure to a recognized antigen. Healthy
986:"Update on the hyper immunoglobulin M syndromes"
1041:Etzioni, Amos; Ochs, Hans D. (1 October 2004).
464:, as well as other treatments, may be needed.
385:. Defective interaction of CD40L-CD40 between
140:(X-linked), characterized by mutations of the
1823:
1398:
1204:Strober, Warren; Gottesman, Susan R. (2014).
99:Allogeneic hematopoietic cell transplantation
8:
1235:Walker's Pediatric Gastrointestinal Disease
2112:Reproductive endocrinology and infertility
1857:
1830:
1816:
1808:
1623:Purine nucleoside phosphorylase deficiency
1471:Transient hypogammaglobulinemia of infancy
1453:
1434:
1405:
1391:
1383:
1265:
1043:"The Hyper IgM SyndromeâAn Evolving Story"
58:
32:
23:
2453:Bachelor of Medicine, Bachelor of Surgery
1176:
1058:
1017:
908:
866:
808:
790:
321:
81:Hyper-IgM syndrome type 1, 2, 3, 4 and 5
500:
1787:Terminal complement pathway deficiency
512:
510:
508:
506:
504:
946:
944:
678:
676:
674:
672:
621:
619:
568:
566:
540:
538:
458:granulocyte colony-stimulating factor
7:
2591:
2352:Physical medicine and rehabilitation
768:
766:
718:
716:
714:
2652:Predominantly antibody deficiencies
2601:
1792:Paroxysmal nocturnal hemoglobinuria
242:, gastrointestinal manifestations,
110:primary immune deficiency disorders
16:Primary immune deficiency disorders
2488:Medical Scientist Training Program
1060:10.1203/01.PDR.0000139318.65842.4A
214:characterized by mutations of the
180:characterized by mutations of the
14:
377:that, when bound to CD40 ligand (
2620:
2610:
2600:
2590:
2581:
2580:
1555:Common variable immunodeficiency
1356:
1002:10.1111/j.1365-2141.2010.08077.x
488:Common variable immunodeficiency
373:is a co-stimulatory receptor on
253:common variable immunodeficiency
2621:
1727:Idiopathic CD4+ lymphocytopenia
2478:Doctor of Osteopathic Medicine
1912:Oral and maxillofacial surgery
1797:Complement receptor deficiency
1667:Adenosine deaminase deficiency
990:British Journal of Haematology
839:Journal of Clinical Immunology
1:
1092:"X-linked hyper IgM syndrome"
953:"X-Linked Hyper IgM Syndrome"
685:"X-Linked Hyper IgM Syndrome"
2647:Syndromes affecting immunity
2458:Bachelor of Medical Sciences
2225:Neurosurgical anesthesiology
910:10.1182/blood-2010-03-274241
1466:X-linked agammaglobulinemia
483:X-linked agammaglobulinemia
2673:
1116:Reference, Genetics Home.
1090:Reference, Genetics Home.
741:10.1016/j.clim.2018.11.007
326:Class switch recombination
188:class switch recombination
2576:
1232:Kleinman, Ronald (2008).
1210:. John Wiley & Sons.
851:10.1007/s10875-016-0291-4
454:antimicrobial prophylaxis
381:), sends a signal to the
225:Hyper-IgM syndrome type 6
211:Hyper-IgM syndrome type 5
203:Hyper-IgM syndrome type 4
177:Hyper-IgM syndrome type 3
159:Hyper-IgM syndrome type 2
137:Hyper-IgM syndrome type 1
40:
31:
2412:Transplantation medicine
2303:Clinical neurophysiology
2220:Obstetric anesthesiology
2140:Interventional radiology
1900:Digestive system surgery
1682:Bare lymphocyte syndrome
1534:WiskottâAldrich syndrome
792:10.3389/fimmu.2018.02172
2283:Intensive care medicine
2257:Mass gathering medicine
2102:Maternalâfetal medicine
1782:Complement 3 deficiency
1767:Complement 4 deficiency
1763:Complement 2 deficiency
1161:10.15252/emmm.202013545
1149:EMBO Molecular Medicine
1122:Genetics Home Reference
1096:Genetics Home Reference
779:Frontiers in Immunology
426:Pulmonary function test
395:humoral immune response
240:pulmonary complications
1875:Cardiothoracic surgery
522:emedicine.medscape.com
391:somatic hyper mutation
327:
263:
262:Pneumocystis pneumonia
192:somatic hyper mutation
91:MRI, Chest radiography
2526:Personalized medicine
2385:Reproductive medicine
2310:Occupational medicine
2264:Evolutionary medicine
1757:Hereditary angioedema
1613:Ataxiaâtelangiectasia
1458:Hypogammaglobulinemia
325:
261:
2546:Traditional medicine
2506:Alternative medicine
2373:Addiction psychiatry
2187:Transfusion medicine
2182:Medical microbiology
2097:Gynecologic oncology
1949:Reproductive surgery
1777:Properdin deficiency
1598:Di George's syndrome
1481:Dysgammaglobulinemia
244:autoimmune disorders
2568:History of medicine
2551:Veterinary medicine
2358:Preventive medicine
2210:Adolescent medicine
2052:Infectious diseases
729:Clinical Immunology
350:underproduction of
337:IgM is the form of
2516:Molecular oncology
2473:Doctor of Medicine
2463:Master of Medicine
2380:Radiation oncology
2252:Emergency medicine
2205:Addiction medicine
2172:Clinical chemistry
2167:Clinical pathology
1959:Transplant surgery
1917:Orthopedic surgery
1895:Colorectal surgery
1629:Hyper IgM syndrome
1539:Hyper-IgE syndrome
1504:Hyper IgM syndrome
1420:disorders causing
1374:Hyper IgM syndrome
1328:External resources
1047:Pediatric Research
462:immunosuppressants
328:
264:
233:Signs and symptoms
106:Hyper IgM syndrome
27:Hyper IgM syndrome
2634:
2633:
2468:Master of Surgery
2432:
2431:
2417:Tropical medicine
2363:Prison healthcare
2278:Hospital medicine
2242:Disaster medicine
2232:Aviation medicine
2047:Hospital medicine
1954:Surgical oncology
1939:Pediatric surgery
1933:
1880:Endocrine surgery
1805:
1804:
1690:
1689:
1589:thymic hypoplasia
1574:T cell deficiency
1568:
1567:
1351:
1350:
903:(26): 5867â5874.
152:isotype switching
117:immunosuppression
103:
102:
87:Diagnostic method
21:Medical condition
2664:
2624:
2623:
2614:
2604:
2603:
2594:
2593:
2584:
2583:
2288:Medical genetics
2273:General practice
2150:Nuclear medicine
2025:Gastroenterology
1981:Vascular surgery
1931:
1858:
1832:
1825:
1818:
1809:
1677:ZAP70 deficiency
1608:Nezelof syndrome
1454:
1435:
1422:immunodeficiency
1407:
1400:
1393:
1384:
1360:
1359:
1266:
1256:
1254:
1252:
1228:
1226:
1224:
1191:
1190:
1180:
1139:
1133:
1132:
1130:
1128:
1113:
1107:
1106:
1104:
1102:
1087:
1081:
1080:
1062:
1038:
1032:
1031:
1021:
981:
975:
974:
973:
971:
948:
939:
938:
912:
887:
881:
880:
870:
829:
823:
822:
812:
794:
770:
761:
760:
720:
709:
707:
705:
703:
680:
667:
666:
664:
662:
648:
642:
641:
639:
637:
623:
614:
613:
611:
609:
595:
589:
588:
586:
584:
570:
561:
560:
558:
556:
542:
533:
532:
530:
528:
514:
73:Chronic diarrhea
63:
62:
42:Immunoglobulin M
36:
24:
2672:
2671:
2667:
2666:
2665:
2663:
2662:
2661:
2637:
2636:
2635:
2630:
2572:
2561:Chief physician
2494:
2439:
2428:
2422:Travel medicine
2407:Sports medicine
2390:Sexual medicine
2330:Palliative care
2325:Pain management
2269:Family medicine
2247:Diving medicine
2193:
2121:
2083:
2076:
1992:
1985:
1944:Plastic surgery
1890:General surgery
1870:Cardiac surgery
1851:
1849:
1841:
1836:
1806:
1801:
1738:
1731:
1719:Lymphocytopenia
1717:
1708:
1686:
1662:
1649:
1638:
1594:hypoparathyroid
1576:
1564:
1543:
1475:
1445:
1424:
1411:
1381:
1380:
1379:
1361:
1357:
1352:
1347:
1346:
1323:
1322:
1277:
1263:
1250:
1248:
1246:
1231:
1222:
1220:
1218:
1203:
1200:
1198:Further reading
1195:
1194:
1141:
1140:
1136:
1126:
1124:
1115:
1114:
1110:
1100:
1098:
1089:
1088:
1084:
1040:
1039:
1035:
983:
982:
978:
969:
967:
950:
949:
942:
889:
888:
884:
831:
830:
826:
772:
771:
764:
722:
721:
712:
701:
699:
682:
681:
670:
660:
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645:
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625:
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617:
607:
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592:
582:
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572:
571:
564:
554:
552:
544:
543:
536:
526:
524:
516:
515:
502:
497:
479:
470:
449:
439:Genetic testing
408:genetic testing
403:
383:B-cell receptor
368:
366:Pathophysiology
343:class switching
320:
235:
129:
57:
22:
17:
12:
11:
5:
2670:
2668:
2660:
2659:
2657:Rare syndromes
2654:
2649:
2639:
2638:
2632:
2631:
2629:
2628:
2618:
2608:
2598:
2588:
2577:
2574:
2573:
2571:
2570:
2565:
2564:
2563:
2553:
2548:
2543:
2538:
2533:
2528:
2523:
2518:
2513:
2508:
2502:
2500:
2499:Related topics
2496:
2495:
2493:
2492:
2491:
2490:
2480:
2475:
2470:
2465:
2460:
2455:
2450:
2448:Medical school
2444:
2442:
2434:
2433:
2430:
2429:
2427:
2426:
2425:
2424:
2414:
2409:
2404:
2402:Sleep medicine
2399:
2398:
2397:
2387:
2382:
2377:
2376:
2375:
2365:
2360:
2355:
2349:
2344:
2343:
2342:
2332:
2327:
2322:
2317:
2312:
2307:
2306:
2305:
2295:
2290:
2285:
2280:
2275:
2266:
2261:
2260:
2259:
2249:
2244:
2239:
2234:
2229:
2228:
2227:
2222:
2215:Anesthesiology
2212:
2207:
2201:
2199:
2195:
2194:
2192:
2191:
2190:
2189:
2184:
2179:
2174:
2169:
2164:
2154:
2153:
2152:
2147:
2145:Neuroradiology
2142:
2131:
2129:
2123:
2122:
2120:
2119:
2114:
2109:
2104:
2099:
2094:
2088:
2086:
2082:Obstetrics and
2078:
2077:
2075:
2074:
2069:
2064:
2059:
2054:
2049:
2044:
2039:
2034:
2033:
2032:
2022:
2017:
2012:
2007:
1997:
1995:
1987:
1986:
1984:
1983:
1978:
1977:
1976:
1966:
1964:Trauma surgery
1961:
1956:
1951:
1946:
1941:
1936:
1935:
1934:
1927:Otolaryngology
1924:
1919:
1914:
1909:
1904:
1903:
1902:
1897:
1887:
1882:
1877:
1872:
1866:
1864:
1855:
1853:subspecialties
1843:
1842:
1837:
1835:
1834:
1827:
1820:
1812:
1803:
1802:
1800:
1799:
1794:
1789:
1784:
1779:
1774:
1772:MBL deficiency
1769:
1760:
1743:
1741:
1733:
1732:
1730:
1729:
1723:
1721:
1710:
1709:
1707:
1706:
1700:
1698:
1692:
1691:
1688:
1687:
1685:
1684:
1679:
1674:
1672:Omenn syndrome
1669:
1653:
1651:
1640:
1639:
1637:
1636:
1617:
1616:
1610:
1601:
1584:
1582:
1570:
1569:
1566:
1565:
1563:
1562:
1557:
1551:
1549:
1545:
1544:
1542:
1541:
1536:
1531:
1525:
1520:
1515:
1510:
1501:
1499:IgM deficiency
1496:
1494:IgG deficiency
1491:
1489:IgA deficiency
1485:
1483:
1477:
1476:
1474:
1473:
1468:
1462:
1460:
1451:
1432:
1426:
1425:
1412:
1410:
1409:
1402:
1395:
1387:
1362:
1355:
1354:
1353:
1349:
1348:
1345:
1344:
1332:
1331:
1329:
1325:
1324:
1321:
1320:
1309:
1294:
1278:
1273:
1272:
1270:
1269:Classification
1262:
1261:External links
1259:
1258:
1257:
1244:
1229:
1216:
1199:
1196:
1193:
1192:
1134:
1108:
1082:
1053:(4): 519â525.
1033:
996:(2): 167â180.
976:
940:
882:
845:(5): 490â501.
824:
762:
710:
668:
643:
615:
590:
562:
534:
499:
498:
496:
493:
492:
491:
485:
478:
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469:
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448:
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443:
440:
437:
434:
428:
423:
417:
402:
399:
367:
364:
319:
316:
315:
314:
313:(degenerative)
311:Encephalopathy
308:
303:
298:
296:Hypothyroidism
293:
287:
281:
234:
231:
230:
229:
221:
207:
199:
173:
155:
128:
125:
101:
100:
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29:
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15:
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2:
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2539:
2537:
2534:
2532:
2531:Public health
2529:
2527:
2524:
2522:
2519:
2517:
2514:
2512:
2511:Allied health
2509:
2507:
2504:
2503:
2501:
2497:
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2320:Oral medicine
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2315:Ophthalmology
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2208:
2206:
2203:
2202:
2200:
2196:
2188:
2185:
2183:
2180:
2178:
2177:Cytopathology
2175:
2173:
2170:
2168:
2165:
2163:
2160:
2159:
2158:
2155:
2151:
2148:
2146:
2143:
2141:
2138:
2137:
2136:
2133:
2132:
2130:
2128:
2124:
2118:
2117:Urogynecology
2115:
2113:
2110:
2108:
2105:
2103:
2100:
2098:
2095:
2093:
2090:
2089:
2087:
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2079:
2073:
2070:
2068:
2065:
2063:
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2045:
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2040:
2038:
2035:
2031:
2028:
2027:
2026:
2023:
2021:
2020:Endocrinology
2018:
2016:
2013:
2011:
2008:
2006:
2002:
1999:
1998:
1996:
1994:
1988:
1982:
1979:
1975:
1972:
1971:
1970:
1967:
1965:
1962:
1960:
1957:
1955:
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1950:
1947:
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1930:
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1925:
1923:
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1901:
1898:
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1873:
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1865:
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1634:
1630:
1627:
1626:
1625:
1624:
1621:
1614:
1611:
1609:
1605:
1604:euparathyroid
1602:
1599:
1595:
1592:
1590:
1586:
1585:
1583:
1580:
1575:
1571:
1561:
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1343:
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1276:
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1267:
1260:
1247:
1245:9781550093643
1241:
1237:
1236:
1230:
1219:
1217:9781118966006
1213:
1209:
1208:
1202:
1201:
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1188:
1184:
1179:
1174:
1170:
1166:
1162:
1158:
1155:(3): e13545.
1154:
1150:
1146:
1138:
1135:
1123:
1119:
1112:
1109:
1097:
1093:
1086:
1083:
1078:
1074:
1070:
1066:
1061:
1056:
1052:
1048:
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1034:
1029:
1025:
1020:
1015:
1011:
1007:
1003:
999:
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987:
980:
977:
966:
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958:
954:
947:
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941:
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920:
916:
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906:
902:
898:
894:
886:
883:
878:
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864:
860:
856:
852:
848:
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840:
836:
833:(July 2016).
828:
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719:
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591:
579:
575:
569:
567:
563:
551:
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523:
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489:
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357:
353:
348:
344:
340:
335:
333:
324:
317:
312:
309:
307:
304:
302:
299:
297:
294:
292:
288:
286:(Hepatitis C)
285:
282:
280:
276:
274:
269:
266:
265:
260:
256:
254:
249:
248:malignancies.
245:
241:
232:
227:
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208:
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200:
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72:
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66:
61:
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52:
50:
46:
43:
39:
35:
30:
25:
19:
2536:Rural health
2521:Nanomedicine
2072:Rheumatology
2003: /
1922:Hand surgery
1907:Neurosurgery
1748:C1-inhibitor
1746:
1663:
1656:
1628:
1619:
1618:
1603:
1593:
1587:
1560:ICF syndrome
1503:
1372:
1371:
1370:profile for
1367:
1335:
1311:
1296:
1281:
1249:. Retrieved
1238:. PMPH-USA.
1234:
1221:. Retrieved
1206:
1152:
1148:
1137:
1125:. Retrieved
1121:
1111:
1099:. Retrieved
1095:
1085:
1050:
1046:
1036:
993:
989:
979:
968:, retrieved
957:GeneReviewsÂź
956:
900:
896:
885:
842:
838:
827:
782:
778:
732:
728:
700:. Retrieved
688:
659:. Retrieved
655:
646:
634:. Retrieved
630:
606:. Retrieved
602:
593:
581:. Retrieved
577:
553:. Retrieved
549:
525:. Retrieved
521:
471:
468:Epidemiology
450:
404:
387:CD4+ T cells
369:
336:
329:
273:Pneumocystis
272:
236:
223:
215:
209:
201:
181:
175:
163:
157:
141:
135:
130:
120:
114:
105:
104:
18:
2606:Wikiproject
2395:Venereology
2340:Neonatology
2237:Dermatology
2092:Gynaecology
2084:gynaecology
2067:Pulmonology
1885:Eye surgery
1847:Specialties
1620:peripheral:
1251:27 November
1223:27 November
1127:27 November
1118:"CD40 gene"
1101:27 November
708:update 2013
702:12 November
689:GeneReviews
636:16 November
608:16 November
583:16 November
555:16 November
527:27 November
421:radiography
301:Neutropenia
170:heavy chain
2641:Categories
2368:Psychiatry
2354:(PM&R)
2347:Phlebology
2335:Pediatrics
2162:Anatomical
2127:Diagnostic
2107:Obstetrics
2057:Nephrology
2042:Hematology
2037:Geriatrics
2030:Hepatology
2015:Cardiology
2005:Immunology
1753:Angioedema
1739:deficiency
1737:Complement
1714:Leukopenia
1664:autosomal:
1418:complement
495:References
431:Lymph node
172:production
108:is a rare
54:Immunology
2556:Physician
2440:education
2298:Neurology
2293:Narcology
2157:Pathology
2135:Radiology
2010:Angiology
1974:Andrology
1657:x-linked:
1337:eMedicine
1169:1757-4676
1069:0031-3998
1010:0007-1048
919:0006-4971
859:0271-9142
801:1664-3224
749:1521-6616
735:: 19â30.
661:2 January
447:Treatment
401:Diagnosis
306:Arthritis
284:Hepatitis
275:pneumonia
268:Infection
96:Treatment
49:Specialty
2586:Category
2062:Oncology
1993:medicine
1991:Internal
1839:Medicine
1704:HIV/AIDS
1696:Acquired
1647:combined
1439:Antibody
1414:Lymphoid
1342:ped/2457
1187:33475257
1077:15319456
1028:20180797
965:20301576
935:33972331
927:20702779
877:27189378
819:30319630
757:53566466
697:20301576
656:omim.org
631:omim.org
603:omim.org
578:omim.org
550:omim.org
477:See also
339:antibody
332:X-linked
291:diarrhea
289:Chronic
196:in vitro
69:Symptoms
2626:Outline
2596:Commons
2541:Therapy
2438:Medical
2001:Allergy
1969:Urology
1862:Surgery
1443:humoral
1430:Primary
1364:Scholia
1318:D053306
1178:7933961
1143:2021).
1019:2855828
970:22 June
868:5039943
810:6168630
375:B cells
347:B cells
279:Bactrim
148:T cells
2616:Portal
2483:MDâPhD
1660:X-SCID
1644:Severe
1366:has a
1307:279.05
1242:
1214:
1185:
1175:
1167:
1075:
1067:
1026:
1016:
1008:
963:
933:
925:
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857:
817:
807:
799:
755:
747:
695:
490:(CVID)
419:Chest
358:, and
143:CD40LG
121:CD40LG
56:
2198:Other
1650:(B+T)
1548:Other
1368:topic
1292:D80.5
931:S2CID
897:Blood
753:S2CID
379:CD40L
318:Cause
165:AICDA
127:Types
78:Types
1416:and
1313:MeSH
1302:9-CM
1253:2016
1240:ISBN
1225:2016
1212:ISBN
1183:PMID
1165:ISSN
1129:2016
1103:2016
1073:PMID
1065:ISSN
1024:PMID
1006:ISSN
972:2023
961:PMID
923:PMID
915:ISSN
873:PMID
855:ISSN
815:PMID
797:ISSN
745:ISSN
704:2016
693:PMID
663:2018
638:2016
610:2016
585:2016
557:2016
529:2016
433:test
371:CD40
190:and
183:CD40
1932:ENT
1850:and
1298:ICD
1283:ICD
1173:PMC
1157:doi
1055:doi
1014:PMC
998:doi
994:149
905:doi
901:116
863:PMC
847:doi
805:PMC
787:doi
737:doi
733:198
415:MRI
360:IgE
356:IgG
352:IgA
217:UNG
2643::
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Text is available under the Creative Commons Attribution-ShareAlike License. Additional terms may apply.
